OBJECTIVE To investigate and analyze the antibiotic usage in infectious disease hospital,and offer(scientific) rule for antibiotic usage.METHODS The retrospective investigation of antibiotics usage in 1991 in-(patients) from Sep 2004 to Mar 2005 was carried out.RESULTS From 1991 patients,909 patients used antibiotics,percentage of antibiotic usage was 45.7%;66.7% patients used one antibiotic,28.6% patients used twice,(4.73%) used triple.From them 29.3% were used for prevention,and 70.85% for treatment;of all the unsuitable use,widen range of the indication,longer(usage),changing frequently,etc were the main causes.CONCLUSIONS Unsuitable usage of antibiotics could be found in our hospital.It is necessary for hospital to strengthen the (management) of antibiotic usage in inpatients.

Objective To observe and assess the effect of different dosages of aspirin on inflammatory biomarkers, hemorheology (platelet aggregation rate) and clinical prognosis in patients with acute coronary syndrome ( ACS). Methods ACS patients were randomly assigned to receive different dosages of aspirin treatment orally. Patients in group A,B and C took 100 mg, 500 mg and 1000 mg of aspirin per day respectively. They were treated and followed-up for 1 year. High-sensitivity C-reactive protein ( hsCRP) , IL-6, tumor necrosis TNFot and platelet aggregation rate were examined and major adverse cardiac events( MACE) were recorded. Results A total of 312 patients with ACS were enrolled in the study. The baseline characteristics of the three groups were not different with respect to age, gender, cardiovascular risk profile, level of inflammatory biomarkers and concomitant treatment before and after randomization. The levels of baseline serum hsCRP, IL-6 and TNFa were higher in subjects of the study as compared with normal reference value (P<0. 05, <0. 05, <0. 01) and they decreased significantly after therapy with 3 different doses of aspirin (detected at 30 days, 6 months and 12 months, P <0. 001 ) , but there were no significant differences among the three groups( P >0. 05) . Rehospitalization , MACE and the change of platelet aggregation ratio were not significantly different among the three groups. The incidence of gastrointestinal complaints was significantly higher in groups B and C than in group A ( P < 0. 05 ). Conclusions The levels of serum inflammatory biomarker increase in patients with ACS. Aspirin therapy may decrease the level of inflammatory markers significantly, but increasing the dosage of aspirin from 100 mg to 1000 mg daily does not decrease the level of inflammatory markers and the clinical MACEs further. However, the incidence of gastrointestinal complaints increase significantly with the increase of aspirin dosage.

OBJECTIVE To explore the effect of the measures of protection and control of nosocomial infection in the period of H1N1 Influenza prevalence.METHODS Established emergency plan and concrete working procedure,enhanced training.Strictly disinfection and isolation and personal protection,supervising and checking were carried out.RESULTS No nosocomial infection happened in our hospital in the diagnosis and treatment of H1N1 Influenza.CONCLUSIONS Carried out general measures of protection and management are the key to provent nosocomial infection.

Objective To explore the clinical features,genetic causes and prognosis of intellectual disability with epilepsy(ID-E)in children.Methods The data of unknown causes of ID-E children(n=40)who were identified in Department of Pediatrics,Xiangya Hospital of Central South University from March 2015 to March 2016 were respectively analyzed,and follow-up studies were performed to investigate the epilepsy control and intellectual deve-lopment.Results Forty unexplained ID-E included 25(62.5％)male,and 34(85.0％)cases were severe intellectual disability patients.The onset age of epilepsy was 0.16 to 8.00 years old,median age was 1.5 years old.Twenty cases(50.0％)had slow electroencephalogram background,and 22 cases(55.0％)had focal spikes.Ten cases(25.0％)had abnormal cranial images,with brain dysplasia or atrophy.Follow-up lasted from 0.58 to 1.58 years,and 19 cases(47.5％)had seizure control.Twenty-five cases(62.5％)had used at least 2 anti-epilepsy drugs during follow-up,and 19 cases(47.5％)had drug refractory epilepsy.Improvement of mental or motor development in epilepsy controlled group and the uncontrolled group were 12 cases(63.2％)and 2 cases(9.5％).There were separately 8 cases(8/40 cases,20.0％)and 3 cases(3/16 cases,18.8％)diagnosed respectively by whole genome-wide analysis of copy number variants(CNVs)and gene-panel whose CNVs test findings were negative.Conclusions ID-E patients of unknown causes have the following clinical features:they were mostly found in male patients with severe intellectual disability,and drug refractory epilepsy patients have rather high percentage;well controlling of epilepsy is useful for improvement of mental and motor development.Genetic analysis is significant for control and prognosis of ID-E patients,and genome-wide CNVs have high positive rates which can be used as first-tier test to detect genetic etiology of ID-E of unknown cause.

Neurodevelopmental disorder (NDD) is a kind of chronic developmental brain dysfunction disease, with strong phenotypic heterogeneity, complex etiology, strong heredity, most of which lack effective drug treatment, and high incidence rate and disability rate. With the advancement of detection technology, more and more mechanisms of NDD have been discovered, driving the progress of its drug treatment. Strengthening research on the pathogenesis of NDD in the future will contribute to the development of better treatments, thereby improving the quality of life and prognosis of patients.

This patient presented with fever,seizure and bulging fontanelle when he was 6-month-old.According to the investigations,white blood cell (WBC),erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) increased significantly,and Streptococcus Pneumonia grew in both blood and cerebrospinal fluid (CSF).He responded to standard antibiotic treatment poorly even it lasted long enough.At the same time,the inflammation seemed to be over-activated,the WBC level was still elevated,high fever continued.Thus they thought of primary immunodeficiency and sent blood sample for gene panel testing (Sanger sequencing) but got negative result.At last,they added steroid together with anti-tuberculosis drug therapy,his temperature as well as the intracranial pressure became better ever since.At the age of 1 year and 1 month,he got another Streptococcus Pneumonia meningitis,while he was still on anti-tuberculosis drug therapy and tapering off steroid.At this time,he presented with coarse hair,hypohidrosis and delayed eruption of teeth,which strongly indicated Anhidrotic Ectodermal Dysplasia with Immunodeficiency (EDA-ID).NEMO is the most common gene responsible for EDA-ID and locates on X chromosome.It has a pseudogene named IKBKGP which locates downstream of NEMO.IKBKGP and NEMO share 3-10 exons with the homology of 99.8％,which makes it difficult to find out most real mutations within NEMO with Sanger sequencing.Then they performed PCR with the primer starting upstream of the shared exons.Finally,they found out the pathogenic mutation [c.505G ＞ C(p.A169P)] of NEMO,which has been reported.This finding led us to make the right diagnosis as well as the proper treatment and the prognosis for this patient.

Objective: To investigate the clinical features, diagnosis and treatment of febrile infection-related epilepsy syndrome (FIRES). Methods: The clinical data of 12 children with FIRES admitted to Xiangya Hospital of Central South University from 2015 to 2018 were retrospectively analyzed. The basic information, clinical manifestations, electroencephalogram, imaging examination, treatment and prognosis were analyzed. Results: Of the 12 patients, 7 were male and 5 were female. The age of onset was (7.0±3.7)years (1.3 year to 13 years). The average hospitalization time (34-86 days, median 52 days). Twelve patients were healthy before the disease, and had fever before convulsion. The interval between fever and seizure was (3.5±1.7)days (1-7 days). The status epilepticus and consciousness deficit were the main clinical manifestations. The electrogram of 8 patients showed status epilepticus when admitted. 12 patients had disturbance of consciousness; the acute episodes were focal seizures (100%, 12/12) and generalized tonic-clonic seizures (41.7%, 5/12). All patients used 3-5 antiepileptic drugs (median 4), all treated with hormones and gamma globulin. 4 patients with ketogenic diet (KD) were treated within 2 weeks of onset, and the average duration from onset to electroencephalogram (EEG) improvement was (19.2±5.0)days. In 8 patients who did not use KD within 2 weeks of onset, the average duration from onset to EEG improvement was (29.9±9.6)days. Conclusions FIRES is more common in normal children with school age. The main manifestation is refractory status epilepticus in the days after acute fever, focal episodes of seizures, anti-epileptic drug resistance. Early initiation of KD produces a favorable prognosis.

Autoimmune encephalitis(AE) is one of the most rapidly developing research fields in pediatric neurology.Previous studies have indicated that delayed-use or non-use of immunotherapy will lead to poor prognosis.Therefore, this article summarizes the current opinion of immunotherapy and prognostic factors for AE in order to provide treatment guidance for clinicians.

Pediatric central nervous system (CNS) immune-related disease is a group of heterogeneous inflammatory conditions mainly affecting CNS. They usually happen in previously healthy individual, with varied clinical manifestations, pathophysiology and genetic changes. Patients require different therapy as well as have different prognosis. Immunotherapy often is helpful to control the disease. With the development of molecular techniques within the recent 10 years, the clinical spectrum and pathogenesis of these neuroinflammatory diseases is being recognized. Further investigations into these diseases are helpful for early diagnosis and targeted immunotherapy, contribute to decrease the mortality and morbidity, then improve the clinical outcome eventually. This study mainly discuss the neuroinflammatory diseases primarily happen in CNS.

Myasthenia gravis (MG) is an autoimmune disease with neuromuscular junction (NMJ) transmission disorder mediated by various antibodies, dependent on cellular immunity, and involved in complement and cytokines. MG is one of the common diseases in pediatric neurology, which is different from adult MG in diagnosis and treatment. However, there is still a lack of accurate and efficient diagnosis and treatment plan for pediatric MG. In recent years, with the development of pathogenesis, targeted diagnosis and treatment of NMJ transmission disorders caused by immune network disorders in immunopathology and pathogenic antibodies is the current main research direction. This article reviews the progress of auxiliary examination and treatment of MG in children.